The article described our own observation of enteropathic acrodermatitis in a girl aged 2 years 3 months born from the second pregnancy, second delivery, at the gestational age of 39 weeks (cesarean section). At gestational age of 20 weeks, prenatal consultation detected doubled left kidney and doubled pelvis of the right kidney with pyeloectasia. The newborn was transferred from the maternity clinic to the clinical perinatal center on the 2nd day after birth. Parents are second cousins to each other. The family has two children, the eldest girl ages 9 years (healthy) and this child. Literature review shows that zinc deficiency plays the main role in the development of this dermatosis. The disease can manifest during the period from the first days of life up to 1.5 years (when the child starts taking artificial feeding, since breast milk includes a zinc-binding ligand, which contributes to better zinc absorption). The main symptoms of the disease include skin and mucous membrane lesions in patients suffering from gastro-intestinal diseases. The rash is usually symmetrical, have sharp contour and irregular shape. These lesions may be similar to clinical presentation of candidiasis, eczema, psoriasis, and bullous epidermolysis. The disease should be also differentiated with persistent pustular Allopo acrodermatitis and dermatitis herpetiformis. The treatment includes zinc drugs: zinc oxide in administered at a dose of 0.03—0.15 g per day. After clinical improvement, maintenance dose of the drug of 8 mg per day is administered and duration of treatment is determined individually. In the aforementioned case, timely initiated adequate treatment of enteropathic acrodermatitis, which lasted more than 2 years, provided no improvement of skin process. In the case of closely related marriages or genetically determined diseases in some other family members, prenatal diagnosis and consultation at an earlier gestational age is required, which would enable a decision on pregnancy termination.